Materials and Methods This is a cross-sectional study, which was performed on 23 patients with CF admitted at the Pediatric Respiratory Ward of Masih Daneshvari Hospital in 2008. The study was approved by the Ethics Committee

of Masih Daneshvari Hospital. Informed consent was obtained from all patients participating in the study. Patients, who were diagnosed as CF based on two positive sweat tests as well as Selleck Transferase inhibitor clinical manifestations compatible with CF, were included the study.20 Those with had negative Sweat Tests and no positive chromosomal analysis were excluded from the study. Four patients had chromosomal analysis test confirming the diagnosis. High resolution computed tomography, PFT, and clinical findings (on the basis of Inhibitors,research,lifescience,medical Shwachman-Kulczycki scoring system) were used. High resolution computed tomography was performed in all patients, whereas PFT was carried out in only 20 of them. The patients under 6 years could not co-operate, and PFT was not performed in them.

Computed tomography scan was obtained in all patients in supine Inhibitors,research,lifescience,medical position in both expiratory and inspiratory phases from lung apex to the below of costophrenic angles. Thin section (1 mm section thickness and 20 mm interval) CT scans were obtained with a spiral CT unit (Siemens SOMATOM Inhibitors,research,lifescience,medical Emotion, KVP 110). The clinical status of all patients was evaluated by Shwachman-Kulczycki scoring system. This system determines the clinical severity of cystic fibrosis by scoring four parameters including general activity, physical examination, nutrition status and radiological findings. All examinations (HRCT, PFT and evaluation Inhibitors,research,lifescience,medical of clinical status) for evaluation each patient were conducted within two weeks. An attending radiologist and two radiology residents reviewed the CT scans using the parameters listed in scoring system, determined the grades of morphologic signs of bronchiectasis, peri-bronchial wall thickening, mucus plugging and air trapping, and calculated the total score. They were not aware of the patients’ clinical Inhibitors,research,lifescience,medical status

and PFT results. In Brody scoring system,16 the severity and extent )central-peripheral) of bronchiectasis, peribronchial wall thickening, mucus plugging and air trapping were evaluated in right upper lobe, left upper lobe, right middle lobe, lingula, right lower lobe and Left lower lobe. Pulmonay function test included forced enough vital capacity (FVC), forced expiratory volume in 1 second (FEV1) and maximum expiratory flow at 50% and 25% of vital capacity. Results were described as the percentage of the predicted values based on reference values of PFT). Patients were divided into four groups including those with FEV1; <40%, FEV1; 40%-59%, FEV1; 60%-80%, and FEV1; >80% based on FEV1 results. Such groups were considered as severe, moderate, mild and normal, respectively. Shwachman-Kulczycki scoring system was used to evaluate the clinical status of all patients.